Pleuropulmonary Blastoma Presenting as an Oncologic Emergency

Oncologic emergency (OE) is a life-threatening condition caused by a tumor or its treatment and may result from severe mass effect on vital organs. Although thoracic OE are most commonly associated with anterior mediastinal tumors, large intrathoracic tumors can also cause critical cardiopulmonary compromise. We report a rare case of pleuropulmonary blastoma (PPB) presenting as a thoracic OE. A three-year-old girl was transferred with severe respiratory distress due to complete right lung collapse and tracheal deviation to the left because of a large intrathoracic tumor. Pre-emptive intubation to secure the airway was avoided due to a concern for respiratory decompensation. However, further investigation, including percutaneous biopsy and contrast-enhanced computed tomography (CT), required sedation and endotracheal intubation, which subsequently led to deterioration of cardiopulmonary function. Because urgent chemotherapy for the reduction of tumor size was not feasible, emergency surgical decompression was performed. A hemi-clamshell thoracotomy allowed prompt exteriorization of the tumor, decompression of the thoracic cavity, and safe right lower lobectomy. Histopathology confirmed pleuropulmonary blastoma type II. The postoperative course was uneventful, and the patient remains disease-free after adjuvant chemotherapy. This case highlights that pleuropulmonary blastoma can present as a fulminant OE and demonstrates that early surgical decompression using a hemi-clamshell approach can be lifesaving in rapidly deteriorating patients.